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Completed

NCT Number: NCT01651507

Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function

this is a multicenter retrospective study on patients with pulmonary LCH who were sequentially evaluated by concomitant lung HRCT and lung function testing. The objectives of this study were: 1) to determine the changes over time of lung function parameters in patients with pulmonary LCH; 2) to compare the variations of HRCT and lung function results during follow-up; 3) to address the respective interest of these investigations for identifying the patients who eventually will experience a progression of their disease.

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Key information

Conditions

Pulmonary Langerhans Cell Histiocytosis Hemic and Lymphatic Diseases Histiocytosis Histiocytosis, Langerhans-Cell Lung Diseases Lung Diseases, Interstitial Lymphatic Diseases Respiratory Tract Diseases

Age range

14 year and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Saint Louis hospital

Paris, 75010, France

About this study

Pulmonary Langerhans cell histiocytosis (pulmonary LCH) is an uncommon disorder, characterized by the accumulation of CD1a+ Langerhans cells (LCs) organized in granulomas that develop in, and destroy the wall of distal bronchioles. In adults the disease occurs predominantly in young smokers from both genders, with a peak incidence at 20-40 yrs of age, although female may be slightly older. High resolution computed tomography (HRCT) of the lung has provided a considerable input for the diagnosis of pulmonary LCH.

We conducted a multicenter retrospective study on patients with pulmonary LCH who were sequentially evaluated by concomitant lung HRCT and lung function testing. The objectives of this study were: 1) to determine the changes over time of lung function parameters in patients with pulmonary LCH; 2) to compare the variations of HRCT and lung function results during follow-up; 3) to address the respective interest of these investigations for identifying the patients who eventually will experience a progression of their disease. Results of this study may help to improve the management of patients with adult pulmonary LCH.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • pulmonary HLP diagnosed between June 1989 and February 2005

Exclusion criteria

  • followed up for less than 6 months
  • with less than 2 lung HRCT and lung function tests at the same time or within a 2 month period

Treatment and study plan

Primary outcomes

  1. Impairment of lung function

    Time frame: 5 years

    a decrease ≥10% of FEV1 or FVC, or decrease ≥15% of DLCO. Conversely, if the FEV1or FVC increased of ≥10%, or DLCO ≥15%

  2. obstructive pattern

    Time frame: 5 years

    FEV1/FVC ratio <70%

  3. restrictive pattern

    Time frame: 5 years

    TLC <80% of predicted value

  4. lung HRCT cystic score

    Time frame: 5 years

    The extent of cystic lesions (including thick- and thin- walled cysts) was assessed for each of the 6 defined lung areas on HRCT and classified as follows: 0 (no cyst); 1 (<25%); 2 (25-49%); 3 (50-75%) and 4 (>75%) of the lung surface analyzed. For the whole lung, the maximal value for cystic HRCT score was of 24

Sponsors and collaborators

Lead sponsor

Assistance Publique - Hôpitaux de Paris

Other

Registry information

Official study title

Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study

Acronym: HLP

Important dates

Study start
1989
Primary completion
2005
Study completion
2010
First posted
Jul 27, 2012
Registry last updated
Apr 18, 2016

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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